Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.

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Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis. / Hasselblatt, Martin; Oyen, Florian; Gesk, Stefan; Kordes, Uwe; Brigitte, Wrede; Bergmann, Markus; Schmid, Hansjörg; Frühwald, Michael C; Schneppenheim, Reinhard; Siebert, Reiner; Paulus, Werner.

In: J NEUROPATH EXP NEUR, Vol. 68, No. 12, 12, 12.2009, p. 1249-1255.

Research output: SCORING: Contribution to journalSCORING: Journal articleResearchpeer-review

Harvard

Hasselblatt, M, Oyen, F, Gesk, S, Kordes, U, Brigitte, W, Bergmann, M, Schmid, H, Frühwald, MC, Schneppenheim, R, Siebert, R & Paulus, W 2009, 'Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.', J NEUROPATH EXP NEUR, vol. 68, no. 12, 12, pp. 1249-1255. https://doi.org/10.1097/NEN.0b013e3181c06a51

APA

Hasselblatt, M., Oyen, F., Gesk, S., Kordes, U., Brigitte, W., Bergmann, M., Schmid, H., Frühwald, M. C., Schneppenheim, R., Siebert, R., & Paulus, W. (2009). Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis. J NEUROPATH EXP NEUR, 68(12), 1249-1255. [12]. https://doi.org/10.1097/NEN.0b013e3181c06a51

Vancouver

Bibtex

@article{5a7108ee6a684b9298e19913b1c4f3b7,
title = "Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.",
abstract = "Atypical teratoid/rhabdoid tumors are malignant embryonal tumors characterized by the presence of rhabdoid cells, genetic alterations affecting the SMARCB1 gene (hSNF5/INI1), and a poor prognosis. Whether INI1 plays a role in the pathogenesis of other central nervous system tumors is uncertain. We report on cases of 2 young children with unusual intracranial nonrhabdoid neuroectodermal tumors within and around the third or fourth ventricle that are characterized by cribriform strands and trabeculae and well-defined epithelial membrane antigen-immunopositive surfaces and show INI1 protein loss. Histological and immunohistochemical features did not correspond to established tumor types, including atypical teratoid/rhabdoid tumors, medulloepithelioma, choroid plexus carcinoma, and ependymoma. Fluorescence in situ hybridization analyses failed to identify chromosomal alterations affecting the SMARCB1 locus, but sequencing revealed a homozygous 4-bp duplication in exon 4 (492duplCCTT) in one of the tumors. Both children responded well to conventional adjuvant therapy protocols and are alive and in complete remission longer than 5 years postoperatively. We suggest that cribriform neuroepithelial tumor (CRINET) is a nonrhabdoid ventricular tumor that shows loss of tumoral INI1 protein and has a relatively favorable prognosis.",
keywords = "Antineoplastic Combined Chemotherapy Protocols, Brain Neoplasms, Child, Preschool, Chromosomal Proteins, Non-Histone, Combined Modality Therapy, DNA-Binding Proteins, Female, Fourth Ventricle, Humans, Immunohistochemistry, In Situ Hybridization, Fluorescence, Infant, Male, Neoplasms, Neuroepithelial, Neurosurgical Procedures, Prognosis, Radiotherapy, Third Ventricle, Transcription Factors",
author = "Martin Hasselblatt and Florian Oyen and Stefan Gesk and Uwe Kordes and Wrede Brigitte and Markus Bergmann and Hansj{\"o}rg Schmid and Fr{\"u}hwald, {Michael C} and Reinhard Schneppenheim and Reiner Siebert and Werner Paulus",
year = "2009",
month = dec,
doi = "10.1097/NEN.0b013e3181c06a51",
language = "English",
volume = "68",
pages = "1249--1255",
journal = "J NEUROPATH EXP NEUR",
issn = "0022-3069",
publisher = "Lippincott Williams and Wilkins",
number = "12",

}

RIS

TY - JOUR

T1 - Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.

AU - Hasselblatt, Martin

AU - Oyen, Florian

AU - Gesk, Stefan

AU - Kordes, Uwe

AU - Brigitte, Wrede

AU - Bergmann, Markus

AU - Schmid, Hansjörg

AU - Frühwald, Michael C

AU - Schneppenheim, Reinhard

AU - Siebert, Reiner

AU - Paulus, Werner

PY - 2009/12

Y1 - 2009/12

N2 - Atypical teratoid/rhabdoid tumors are malignant embryonal tumors characterized by the presence of rhabdoid cells, genetic alterations affecting the SMARCB1 gene (hSNF5/INI1), and a poor prognosis. Whether INI1 plays a role in the pathogenesis of other central nervous system tumors is uncertain. We report on cases of 2 young children with unusual intracranial nonrhabdoid neuroectodermal tumors within and around the third or fourth ventricle that are characterized by cribriform strands and trabeculae and well-defined epithelial membrane antigen-immunopositive surfaces and show INI1 protein loss. Histological and immunohistochemical features did not correspond to established tumor types, including atypical teratoid/rhabdoid tumors, medulloepithelioma, choroid plexus carcinoma, and ependymoma. Fluorescence in situ hybridization analyses failed to identify chromosomal alterations affecting the SMARCB1 locus, but sequencing revealed a homozygous 4-bp duplication in exon 4 (492duplCCTT) in one of the tumors. Both children responded well to conventional adjuvant therapy protocols and are alive and in complete remission longer than 5 years postoperatively. We suggest that cribriform neuroepithelial tumor (CRINET) is a nonrhabdoid ventricular tumor that shows loss of tumoral INI1 protein and has a relatively favorable prognosis.

AB - Atypical teratoid/rhabdoid tumors are malignant embryonal tumors characterized by the presence of rhabdoid cells, genetic alterations affecting the SMARCB1 gene (hSNF5/INI1), and a poor prognosis. Whether INI1 plays a role in the pathogenesis of other central nervous system tumors is uncertain. We report on cases of 2 young children with unusual intracranial nonrhabdoid neuroectodermal tumors within and around the third or fourth ventricle that are characterized by cribriform strands and trabeculae and well-defined epithelial membrane antigen-immunopositive surfaces and show INI1 protein loss. Histological and immunohistochemical features did not correspond to established tumor types, including atypical teratoid/rhabdoid tumors, medulloepithelioma, choroid plexus carcinoma, and ependymoma. Fluorescence in situ hybridization analyses failed to identify chromosomal alterations affecting the SMARCB1 locus, but sequencing revealed a homozygous 4-bp duplication in exon 4 (492duplCCTT) in one of the tumors. Both children responded well to conventional adjuvant therapy protocols and are alive and in complete remission longer than 5 years postoperatively. We suggest that cribriform neuroepithelial tumor (CRINET) is a nonrhabdoid ventricular tumor that shows loss of tumoral INI1 protein and has a relatively favorable prognosis.

KW - Antineoplastic Combined Chemotherapy Protocols

KW - Brain Neoplasms

KW - Child, Preschool

KW - Chromosomal Proteins, Non-Histone

KW - Combined Modality Therapy

KW - DNA-Binding Proteins

KW - Female

KW - Fourth Ventricle

KW - Humans

KW - Immunohistochemistry

KW - In Situ Hybridization, Fluorescence

KW - Infant

KW - Male

KW - Neoplasms, Neuroepithelial

KW - Neurosurgical Procedures

KW - Prognosis

KW - Radiotherapy

KW - Third Ventricle

KW - Transcription Factors

U2 - 10.1097/NEN.0b013e3181c06a51

DO - 10.1097/NEN.0b013e3181c06a51

M3 - SCORING: Journal article

C2 - 19915490

VL - 68

SP - 1249

EP - 1255

JO - J NEUROPATH EXP NEUR

JF - J NEUROPATH EXP NEUR

SN - 0022-3069

IS - 12

M1 - 12

ER -